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List of works by Carla M. Koehler

A Chemical Biology Approach to Model Pontocerebellar Hypoplasia Type 1B (PCH1B).

scientific article published on 6 September 2018

A mutation in PNPT1, encoding mitochondrial-RNA-import protein PNPase, causes hereditary hearing loss

scientific article

A new function in translocation for the mitochondrial i-AAA protease Yme1: import of polynucleotide phosphorylase into the intermembrane space

scientific article

A role for cytochrome c and cytochrome c peroxidase in electron shuttling from Erv1.

scientific article

A small molecule inhibitor of redox-regulated protein translocation into mitochondria

scientific article

Adaptation of a Genetic Screen Reveals an Inhibitor for Mitochondrial Protein Import Component Tim44.

scientific article

Assembly of the three small Tim proteins precedes docking to the mitochondrial carrier translocase

scientific article

Atomic structure of a toxic, oligomeric segment of SOD1 linked to amyotrophic lateral sclerosis (ALS).

scientific article published on 31 July 2017

Barth syndrome mutations that cause tafazzin complex lability ⬇️

scientific article published on February 7, 2011

Cardiolipin defines the interactome of the major ADP/ATP carrier protein of the mitochondrial inner membrane

scientific article published on September 2008

Cardiolipin defines the interactome of the major ADP/ATP carrier protein of the mitochondrial inner membrane.

scientific article published in June 2012

Correcting human mitochondrial mutations with targeted RNA import ⬇️

scientific article published on March 12, 2012

Defective mitochondrial protein translocation precludes normal Caenorhabditis elegans development

scientific article published on 13 October 2004

Defining functional classes of Barth syndrome mutation in humans

scientific article

Defining the role of oxygen tension in human neural progenitor fate

scientific article published on 30 October 2014

ER-mitochondria contacts: Actin dynamics at the ER control mitochondrial fission via calcium release.

scientific article published on 19 December 2017

FAM210B is an erythropoietin target and regulates erythroid heme synthesis by controlling mitochondrial iron import and ferrochelatase activity

scientific article published in Journal of Biological Chemistry

Hereditary spastic paraplegia: respiratory choke or unactivated substrate?

scientific article published in October 2005

Human deafness dystonia syndrome is caused by a defect in assembly of the DDP1/TIMM8a-TIMM13 complex

scientific article (publication date: March 2002)

Human polynucleotide phosphorylase: location matters

scientific article published on 5 November 2007

Importing mitochondrial proteins: machineries and mechanisms

scientific article (publication date: 21 August 2009)

Loss of function of SLC25A46 causes lethal congenital pontocerebellar hypoplasia.

scientific article

Macrocytic anemia and mitochondriopathy resulting from a defect in sideroflexin 4.

scientific article published on 10 October 2013

Mammalian polynucleotide phosphorylase is an intermembrane space RNase that maintains mitochondrial homeostasis

scientific article

Measuring energy metabolism in cultured cells, including human pluripotent stem cells and differentiated cells.

scientific article published on 10 May 2012

Metabolic Regulation in Pluripotent Stem Cells during Reprogramming and Self-Renewal ⬇️

scientific article published on November 2, 2012

Mia40 Protein Serves as an Electron Sink in the Mia40-Erv1 Import Pathway

scientific article

Mitochondria-targeted RNA import

scientific article

Mitochondrial Transfer by Photothermal Nanoblade Restores Metabolite Profile in Mammalian Cells.

scientific article published on May 2016

Mitochondrial mislocalization and altered assembly of a cluster of Barth syndrome mutant tafazzins

scientific article

Osm1 facilitates the transfer of electrons from Erv1 to fumarate in the redox-regulated import pathway in the mitochondrial intermembrane space

scientific article published on 16 August 2017

P53 Regulates Rapid Apoptosis in Human Pluripotent Stem Cells.

scientific article published on 31 July 2015

PNPASE and RNA trafficking into mitochondria ⬇️

scientific article published on October 13, 2011

PNPASE regulates RNA import into mitochondria

scientific article

PNPase knockout results in mtDNA loss and an altered metabolic gene expression program

scientific article published on 19 July 2018

Patterns that define the four domains conserved in known and novel isoforms of the protein import receptor Tom20

scientific article (publication date: 18 March 2005)

Pharmacologic rescue of an enzyme-trafficking defect in primary hyperoxaluria 1.

scientific article

Rapid degradation of mutant SLC25A46 by the ubiquitin-proteasome system results in MFN1/2-mediated hyperfusion of mitochondria

scientific article published on 05 January 2017

Reconstitution of the mia40-erv1 oxidative folding pathway for the small tim proteins

scientific article published on 28 May 2009

Redox pathways of the mitochondrion

scientific article published on May 2006

Redox regulation of protein folding in the mitochondrial intermembrane space

scientific article

Repurposing Approach Identifies Auranofin with Broad Spectrum Antifungal Activity That Targets Mia40-Erv1 Pathway

scientific article

Role of twin Cys-Xaa9-Cys motif cysteines in mitochondrial import of the cytochrome C oxidase biogenesis factor Cmc1.

scientific article

Stendomycin selectively inhibits TIM23-dependent mitochondrial protein import.

scientific article published on 9 October 2017

Substrate specificity of the TIM22 mitochondrial import pathway revealed with small molecule inhibitor of protein translocation

scientific article

TMEM14C is required for erythroid mitochondrial heme metabolism

scientific journal article

The TCL1 oncoprotein binds the RNase PH domains of the PNPase exoribonuclease without affecting its RNA degrading activity

scientific article

The Taz1p transacylase is imported and sorted into the outer mitochondrial membrane via a membrane anchor domain.

scientific article published on 27 September 2013

The Tim8–Tim13 Complex Has Multiple Substrate Binding Sites and Binds Cooperatively to Tim23

scientific article published on 24 October 2008

The Tim9p-Tim10p complex binds to the transmembrane domains of the ADP/ATP carrier

scientific article

The calcium-binding aspartate/glutamate carriers, citrin and aralar1, are new substrates for the DDP1/TIMM8a-TIMM13 complex

scientific article

The cardiolipin transacylase, tafazzin, associates with two distinct respiratory components providing insight into Barth syndrome

scientific article

The role of Hot13p and redox chemistry in the mitochondrial TIM22 import pathway

scientific article

The role of Tim9p in the assembly of the TIM22 import complexes

scientific article published in March 2003

The role of the Tim8p-Tim13p complex in a conserved import pathway for mitochondrial polytopic inner membrane proteins

scientific article

Tim54p connects inner membrane assembly and proteolytic pathways in the mitochondrion

scientific article

UCP2 regulates energy metabolism and differentiation potential of human pluripotent stem cells

scientific article published on April 2016

UCP2 regulates energy metabolism and differentiation potential of human pluripotent stem cells.

scientific article published on 15 November 2011

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